Case of the Day : Sclerotic Sphenoid lesion

Sclerotic Sphenoid Lesion

Patient is a middle aged female with headache.

DIAGNOSIS : FIBROUS DYSPLASIA of Sphenoid bone (right).

Sclerotic lesion with the characteristic / diagnostic 'Ground Glass matrix' is seen here involving the sphenoid body and  lesser wing mainly of the right side. Lesion is completely involving the right optic canal, with no narrowing of the canal.

Polyostotic Fibrous Dysplasia - is a feature of McCune Albright Syndrome - associated with precocious puberty and pigmentation. MA-Syndrome usually have Cafe-au-lait patches, which is described to have a 'Coast of Maine' appearance - irregular margins (c.f 'Coast of California' appearance of Cafe-au-lait patches of neurofibromatosis).

Polyostotic fibrous dysplasia having a rare association of intramuscular myxomas is seen in Mazabraud Syndrome.